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Background Survivors of childhood brain tumors or other acquired brain injury (ABI) are at risk of poor health-related quality of life (HRQoL); its valid and reliable assessment is essential to evaluate the effect of their illness on their lives. The aim of this review was to critically appraise psychometric properties of patient-reported outcome measures (PROMs) of HRQoL for these children, to be able to make informed decisions about the most suitable PROM for use in clinical practice. Methods We searched MEDLINE, EMBASE, and PsycINFO for studies evaluating measurement properties of HRQoL PROMs in children treated for brain tumors or other ABI. Methodological quality of relevant studies was evaluated using the consensus-based standards for the selection of health status measurement instruments checklist. Results Eight papers reported measurement properties of 4 questionnaires: Health Utilities Index (HUI), PedsQL Core and Brain Tumor Modules, and Child and Family Follow-up Survey (CFFS). Only the CFFS had evidence of content and structural validity. It also demonstrated good internal consistency, whereas both PedsQL modules had conflicting evidence regarding this. Conflicting evidence regarding test-retest reliability was reported for the HUI and PedsQL Core Module only. Evidence of measurement error/precision was favorable for HUI and CFFS and absent for both PedsQL modules. All 4 PROMs had some evidence of construct validity/hypothesis testing but no evidence of responsiveness to change. Conclusions Valid and reliable assessment is essential to evaluate impact of ABI on young lives. However, measurement properties of PROMs evaluating HRQoL appropriate for this population require further evaluation, specifically construct validity, internal consistency, and responsiveness to change. [ABSTRACT FROM AUTHOR]

We examined phonological recoding during silent sentence reading in teenagers with a history of dyslexia and their typically developing peers. Two experiments are reported in which participants' eye movements were recorded as they read sentences containing correctly spelled words (e.g., church), pseudohomophones (e.g., cherch), and spelling controls (e.g., charch). In Experiment 1 we examined foveal processing of the target word/nonword stimuli, and in Experiment 2 we examined parafoveal pre-processing. There were four participant groups–older teenagers with a history of dyslexia, older typically developing teenagers who were matched for age, younger typically developing teenagers who were matched for reading level, and younger teenagers with a history of dyslexia. All four participant groups showed a pseudohomophone advantage, both from foveal processing and parafoveal pre-processing, indicating that teenagers with a history of dyslexia engage in phonological recoding for lexical identification during silent sentence reading in a comparable manner to their typically developing peers. [ABSTRACT FROM AUTHOR]

Abstract: There has been considerable variability within the literature concerning the extent to which deaf/hard of hearing individuals are able to process phonological codes during reading. Two experiments are reported in which participants’ eye movements were recorded as they read sentences containing correctly spelled words (e.g., church), pseudohomophones (e.g., cherch), and spelling controls (e.g., charch). We examined both foveal processing and parafoveal pre‐processing of phonology for three participant groups—teenagers with permanent childhood hearing loss (PCHL), chronological age‐matched controls, and reading age‐matched controls. The teenagers with PCHL showed a pseudohomophone advantage from both directly fixated words and parafoveal preview, similar to their hearing peers. These data provide strong evidence for phonological recoding during silent reading in teenagers with PCHL. [ABSTRACT FROM AUTHOR]

Background: Sleep-disordered breathing (SDB) is often co-morbid with conductive hearing loss in early childhood due to a shared aetiology of adenotonsillar hypertrophy. Hearing loss is independently associated with impairment of executive function and behavioural difficulties. We hypothesised that these impairments in children with SDB may be mediated through hearing loss.Methods: Fifty-eight children including 37 snorers awaiting adenotonsillectomy and 21 healthy non-snoring controls, aged 3-5 years, were assessed with pure tone audiometry, Strengths and Difficulties (SDQ), Behaviour Rating of Executive Function (BRIEF-P), and Childhood Middle Ear Disease and Hearing questionnaires. Polysomnography in snoring children generated an obstructive apnoea/hypopnea index (OAHI). Two regression models examined the effect of SDB and the mediating impact of hearing loss on BRIEF and SDQ.Results: Snoring children had significantly poorer hearing, greater past exposure to hearing loss, and higher total SDQ and BRIEF-P scores than non-snoring controls. The first regression model, including all children, demonstrated that the impact of snoring on BRIEF_P, but not SDQ, was entirely mediated by a history of hearing loss exposure but not same-day audiometry. The second model examined snoring children only, categorising the group into 12 with obstructive sleep apnoea (OSA) (OAHI ≥ 5) and 25 without OSA. OSA had a direct effect on SDQ scores, but this was not mediated by a history of hearing loss.Conclusion: In early childhood, conductive hearing loss mediates the relationship between SDB, irrespective of severity, and parent report of executive function but not behaviour. Treatment of hearing loss in pre-school SDB might improve executive function. [ABSTRACT FROM AUTHOR]

Objective: To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment. Methods: Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (ICD‐10). Results: A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% confidence interval [CI] 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% CI 48% to 62%, chi‐square 4.3, P = .037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% CI 1.1 to 23.6, P = .037). Twenty of 37 (54%, 95% CI 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% CI 52% to 66%, chi‐square 0.35, P = .55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead‐time into account (odds ratio 0.8, 95% CI 0.4 to 1.7, P = .62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy. Significance: This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment. [ABSTRACT FROM AUTHOR]

Background: Infantile spasms constitutes a severe infantile epilepsy syndrome that is difficult to treat and has a high morbidity. Hormonal therapies or vigabatrin are the most commonly used treatments. We aimed to assess whether combining the treatments would be more effective than hormonal therapy alone.Methods: In this multicentre, open-label randomised trial, 102 hospitals (Australia [three], Germany [11], New Zealand [two], Switzerland [three], and the UK [83]) enrolled infants who had a clinical diagnosis of infantile spasms and a hypsarrhythmic (or similar) EEG no more than 7 days before enrolment. Participants were randomly assigned (1:1) by a secure website to receive hormonal therapy with vigabatrin or hormonal therapy alone. If parents consented, there was an additional randomisation (1:1) of type of hormonal therapy used (prednisolone or tetracosactide depot). Block randomisation was stratified for hormonal treatment and risk of developmental impairment. Parents and clinicians were not masked to therapy, but investigators assessing electro-clinical outcome were masked to treatment allocation. Minimum doses were prednisolone 10 mg four times a day or intramuscular tetracosactide depot 0·5 mg (40 IU) on alternate days with or without vigabatrin 100 mg/kg per day. The primary outcome was cessation of spasms, which was defined as no witnessed spasms on and between day 14 and day 42 from trial entry, as recorded by parents and carers in a seizure diary. Analysis was by intention to treat. The trial is registered with The International Standard Randomised Controlled Trial Number (ISRCTN), number 54363174, and the European Union Drug Regulating Authorities Clinical Trials (EUDRACT), number 2006-000788-27.Findings: Between March 7, 2007, and May 22, 2014, 766 infants were screened and, of those, 377 were randomly assigned to hormonal therapy with vigabatrin (186) or hormonal therapy alone (191). All 377 infants were assessed for the primary outcome. Between days 14 and 42 inclusive no spasms were witnessed in 133 (72%) of 186 patients on hormonal therapy with vigabatrin compared with 108 (57%) of 191 patients on hormonal therapy alone (difference 15·0%, 95% CI 5·1-24·9, p=0·002). Serious adverse reactions necessitating hospitalisation occurred in 33 infants (16 on hormonal therapy alone and 17 on hormonal therapy with vigabatrin). The most common serious adverse reaction was infection occurring in five infants on hormonal therapy alone and four on hormonal therapy with vigabatrin. There were no deaths attributable to treatment.Interpretation: Hormonal therapy with vigabatrin is significantly more effective at stopping infantile spasms than hormonal therapy alone. The 4 week period of spasm cessation required to achieve a primary clinical response to treatment suggests that the effect seen might be sustained, but this needs to be confirmed at the 18 month follow-up.Funding: The Castang Foundation, Bath Unit for Research in Paediatrics, National Institute of Health Research, the Royal United Hospitals Bath NHS Foundation Trust, the BRONNER-BENDUNG Stifung/Gernsbach, and University Children's Hospital Zurich. [ABSTRACT FROM AUTHOR]

Objective: To determine whether the benefits of universal newborn hearing screening (UNHS) seen at age 8 years persist through the second decade.Design: Prospective cohort study of a population sample of children with permanent childhood hearing impairment (PCHI) followed up for 17 years since birth in periods with (or without) UNHS.Setting: Birth cohort of 100 000 in southern England.Participants: 114 teenagers aged 13-19 years, 76 with PCHI and 38 with normal hearing. All had previously their reading assessed aged 6-10 years.Interventions: Birth in periods with and without UNHS; confirmation of PCHI before and after age 9 months.Main Outcome Measure: Reading comprehension ability. Regression modelling took account of severity of hearing loss, non-verbal ability, maternal education and main language.Results: Confirmation of PCHI by age 9 months was associated with significantly higher mean z-scores for reading comprehension (adjusted mean difference 1.17, 95% CI 0.36 to 1.97) although birth during periods with UNHS was not (adjusted mean difference 0.15, 95% CI -0.75 to 1.06). The gap between the reading comprehension z-scores of teenagers with early compared with late confirmed PCHI had widened at an adjusted mean rate of 0.06 per year (95% CI -0.02 to 0.13) during the 9.2-year mean interval since the previous assessment.Conclusions: The benefit to reading comprehension of confirmation of PCHI by age 9 months increases during the teenage years. This strengthens the case for UNHS programmes that lead to early confirmation of permanent hearing loss.Trial Registration Number: ISRCTN03307358. [ABSTRACT FROM AUTHOR]

Background We identified child-related determinants of health-related quality of life (HRQoL) in children aged 8–14 years who were treated for 2 common types of pediatric brain tumors. Methods Questionnaire measures of HRQoL and psychometric assessments were completed by 110 children on 3 occasions over 24 months. Of these 110, 72 were within 3 years of diagnosis of a cerebellar tumor (37 standard-risk medulloblastoma, 35 low-grade cerebellar astrocytoma), and 38 were in a nontumor group. HRQoL, executive function, health status, and behavioral difficulties were also assessed by parents and teachers as appropriate. Regression modeling was used to relate HRQoL z scores to age, sex, socioeconomic status, and 5 domains of functioning: Cognition, Emotion, Social, Motor and Sensory, and Behavior. Results HRQoL z scores were significantly lower after astrocytoma than those in the nontumor group and significantly lower again in the medulloblastoma group, both by self-report and by parent-report. In regression modeling, significant child-related predictors of poorer HRQoL z scores by self-report were poorer cognitive and emotional function (both z scores) and greater age (years) at enrollment (B = 0.038, 0.098, 0.136, respectively). By parent-report, poorer cognitive, emotional and motor or sensory function (z score) were predictive of lower subsequent HRQoL of the child (B = 0.043, 0.112, 0.019, respectively), while age at enrollment was not. Conclusions Early screening of cognitive and emotional function in this age group, which are potentially amenable to change, could identify those at risk of poor HRQoL and provide a rational basis for interventions to improve HRQoL. [ABSTRACT FROM PUBLISHER]